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Perinuclear Antineutrophil Cytoplasmic Antibody-Associated Ocular Manifestations: Case Series and Literature Review
1Department of Ophthalmology, Queen Elizabeth Hospital, Kota Kinabalu, Malaysia.
Purpose:
To investigate the diversity of ocular manifestations in patients with positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA).
Methods:
The clinical records of five patients exhibiting ocular manifestations and testing positive for serum MPO-ANCA were retrospectively reviewed.
Results:
Three out of five patients were female. The youngest patient was aged 26, whereas the eldest was 83 years old. 80% (n = 4) of them had purely anterior segment involvement, with the commonest manifestation being peripheral ulcerative keratitis and scleritis. Only one patient had posterior segment involvement, specifically posterior ischemic optic neuropathy. Two patients demonstrated unilateral involvement. There was only one patient diagnosed with systemic involvement, presenting as mixed nephrotic and nephritic syndrome, who required a course of intravenous methylprednisolone during the active stage. Three patients required second-line immunosuppressants throughout the course due to frequent relapse.
Conclusion:
Anterior segment ocular manifestations are not uncommon and can be the initial presentation of p-ANCA-associated vasculitis (AAV). Therefore, its evaluation should be considered in establishing the diagnosis of AAV, a life-threatening disease.
Insights
Ocular manifestations, particularly peripheral ulcerative keratitis and scleritis, are common in patients with perinuclear antineutrophil cytoplasmic antibody (p-ANCA)-associated vasculitis. Early evaluation of these eye conditions is crucial for diagnosing this serious disease.
Area of Science:
- Ophthalmology
- Rheumatology
- Immunology
Background:
- Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) is associated with systemic vasculitis.
- Ocular involvement can be a significant feature of ANCA-associated vasculitis (AAV).
Purpose of the Study:
- To explore the range of eye conditions in patients with positive p-ANCA.
- To understand the clinical presentation of ocular manifestations in p-ANCA positive individuals.
Main Methods:
- Retrospective review of clinical records.
- Analysis of five patients with ocular symptoms and positive MPO-ANCA.
Main Results:
- 80% of patients had anterior segment involvement, primarily peripheral ulcerative keratitis and scleritis.
- One patient experienced posterior segment disease (ischemic optic neuropathy).
- Most cases involved unilateral ocular manifestations; systemic disease was rare.
Conclusions:
- Anterior segment ocular issues are frequent and can be the first sign of p-ANCA-associated vasculitis.
- Ophthalmological assessment is vital for diagnosing AAV, a potentially life-threatening condition.
